AB1305 A SYSTEMATIC REVIEW OF AA AMYLOIDOSIS AMONG PATIENTS WITH BEHÇET’S SYNDROME
Authors: G. Karatemiz, S. N. Esatoglu, M. Gurcan, Y. Ozguler, S. Yurdakul, V. Hamuryudan, I. Fresko, M. Melikoglu, E. Seyahi, S. Ugurlu, H. Ozdogan, H. Yazici, G. Hatemi
Journal: Annals of the Rheumatic Diseases (2022)

Background Data on patients with Behçet’s syndrome (BS) complicated with AA amyloidosis is limited to case reports or case series with a small number of patients. Objectives In this study, we aimed to perform a systematic review (SR) of published reports on BS patients with AA amyloidosis. Methods PubMed and EMBASE were searched with the keywords “Behcet* AND amyloidosis”, without date and language restriction, until May 2020. Two independent reviewers (SNE, GK) performed title/abstract and full text screening and data extraction. A third reviewer (GH) made the final decision in case of disagreement between the two reviewers. Studies that reported patients who were reported by authors as having BS and AA amyloidosis were included. The risk of bias assessment was done using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) tool. Results The systematic literature search yielded 760 articles of which 703 were excluded after title and abstract review. After full-text review, we further excluded 15 duplicate articles and 1 article was added after handsearching the reference lists of the full texts. Finally, we included 43 articles reporting 96 cases. Among these articles, 38 were case reports and 5 were case series reporting between 6 and 14 patients. All patients but 8 were reported from Mediterranean countries. The quality of all articles according to GRADE was very low due to the lack of a control group. The main features of the patients were male predominance (81/96, 84%), a high frequency of major organ involvement (62/80, 77.5%) especially vascular involvement (60%), a low frequency of comorbidities predisposing to AA amyloidosis (11/96, 11.5%), and a very low frequency of gastrointestinal involvement (3/72, 4%). All but 8 patients were diagnosed with BS and AA amyloidosis simultaneously. The most common presentation was nephrotic syndrome (60/81, 74%). Presenting symptoms other than proteinuria were diarrhea (n=2), acute renal failure (n=2), upper gastrointestinal bleeding (n=1), end stage renal disease (ESRD) (n=1), cardiac symptoms due to cor pulmonale (n=1), and hypertension (n=1). Renal biopsy (72%) and rectal biopsy (17%) were the most commonly used procedures to diagnose AA amyloidosis. After diagnosing AA amyloidosis, colchicine was initiated in 58 patients, cyclophosphamide in 16, and biologics in 3 (1 anakinra and 2 tocilizumab). In the 67 patients with available data on follow-up, 43% of the patients were followed-up for ≤1 year and median follow-up duration was 20 months (IQR: 4-48). Among the 64 patients with available data, 30 (47%) had developed ESRD. Among the 72 patients with available data on survival status, 30 patients (42%) had died. Ten patients (33%) had died within 6 months, 15 had died after a median follow-up of 48 months (IQR: 24-150), and follow-up duration was not available in the remaining 5 patients including 3 patients whose diagnoses were made by autopsy. Reasons for death were infection (n=7), ESRD (n=6), intractable diarrhea (n=3), pulmonary embolism (n=1), cor pulmonale (n=1), hemorrhage due to pulmonary artery aneurysm (n=1), liver cirrhosis (n=1), gastric cancer (n=1), subarachnoid hemorrhage (n=1), and not reported (n=8). Conclusion Male gender and major organ involvement, especially vascular involvement, appear to be risk factors for the development of AA amyloidosis in BS patients. While BS patients complicated with AA amyloidosis have been reported rarely, it is a fatal complication of BS. One third of the patients had died within 6 months after AA amyloidosis diagnosis. Disclosure of Interests Güzin Karatemiz: None declared, Sinem Nihal Esatoglu Speakers bureau: Sinem Nihal Esatoglu has received honorariums for presentations from UCB Pharma, Roche, Pfizer, and Merck Sharp Dohme., Mert Gurcan: None declared, Yesim Ozguler Speakers bureau: Yesim Ozguler has received honorariums for presentations from UCB Pharma, Novartis, and Pfizer., Sebahattin Yurdakul: None declared, Vedat Hamuryudan Speakers bureau: Vedat Hamuryudan has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma., Grant/research support from: Vedat Hamuryudan has received grant/research support from Celgene., Izzet Fresko: None declared, Melike Melikoglu: None declared, Emire Seyahi Speakers bureau: Emire Seyahi has received honorariums for presentations from Novartis, Pfizer, AbbVie, and Gliead., Serdal Ugurlu: None declared, Huri Ozdogan: None declared, Hasan Yazici: None declared, Gulen Hatemi Speakers bureau: Gulen Hatemi has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma, Grant/research support from: Gulen Hatemi has received grant/research support from Celgene.